A case of cerebrotendinous xanthomatosis. II: The sterol content of a cataractous lens.
The cholestanol content of a cataractous lens nucleus from a patient with cerebrotendinous xanthomatosis (CTX) was quantified by gas chromatography-mass spectrometry and found to be 0.27 micrograms per mg freeze-dried lens tissue. The cholestanol-cholesterol ratio of 1.7% in the lens nucleus was similar to that in the serum of the CTX patient. The cholestanol content and cholestanol-cholesterol ratio in the CTX lens were approximately four-fold and six-fold greater respectively than the mean levels found in three senile cataractous lens nuclei analysed simultaneously for comparative purposes.
McKenna
P
P
Department of Clinical Biochemistry, Royal Victoria Infirmary, Newcastle upon Tyne.
Morgan
S J
SJ
Bosanquet
R C
RC
Laker
M F
MF
D000328:Adult; D000368:Aged; D001927:Brain Diseases / Q000378:metabolism; D002386:Cataract / Q000150:complications / Q000378:metabolism*; D004083:Cholestanol / Q000378:metabolism*; D002784:Cholesterol / Q000378:metabolism; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D007907:Lens Nucleus, Crystalline / Q000378:metabolism*; D008297:Male; D013710:Tendons; D014973:Xanthomatosis / Q000150:complications / Q000378:metabolism*
D004083:Cholestanol; D002784:Cholesterol